乳腺低度恶性导管周间质肉瘤临床病理观察

Clinicopathological characteristics of low-grade periductal stromal sarcoma of breast

探讨乳腺低度恶性导管周间质肉瘤的临床病理特点。方法通过HE及免疫组化染色对1例罕见的低级别导管周间质肉瘤进行临床病理观察。结果乳腺导管周间质肉瘤(低度恶性)具有独特的镜下特点:肿瘤由梭形肉瘤样细胞围绕开放的腺管和导管增生形成"袖套样"结构,无叶状结构;纤维或纤维脂肪组织将病变分隔成多个结节;病变浸润周围乳腺组织;可见骨化灶。免疫表型:肿瘤细胞vimentin弥漫强(+),CD99和bcl-2(+),SMA和CD34灶状(+),CD10及p53仅个别瘤细胞(+),Ki-67阳性率80%,广谱CK、EMA、ER、PR、S-100、CD117和desmin、p16均为(-)。该病例行乳腺改良根治术,无其他辅助治疗,随访无复发。结论导管周间质肉瘤是一种低度恶性肿瘤,首选治疗方式为手术切除,术中保证足够的正常乳腺组织切缘以避免复发。因治疗方式不同,需要与其他乳腺梭形细胞肿瘤相鉴别。

Purpose To explore the clinicopathological characteristics of periductal stromal sarcoma. Methods One case of periductal stromal sarcoma was studied using HE and immunohistochemical staining. Results The histologic features peculiarly of periductal stromal sarcoma（ low-grade） were as follows： the tumor was composed of sarcomatous spindle cells that formed cuffs around multiple open tubule and ducts,devoid of the phyllodes pattern. Multiple nodules were separated by fiber and adipose tissue. The tumor infiltrated surrounding breast tissue,with local sclerotization.Immunohistochemistry showed that the tumor cells were strong diffusely positive for vimentin,CD99,bcl-2,focally positive for smooth muscle actin,CD34,less reactive for CD10,p53,Ki-67 staining was about 80%,and negative for cytokeratin,EMA,ER,PR,S-100,CD117,desmin and p16. The patient underwent simplified radical mastectomy. No adjuvant examination was given. At 36 months of close follow-up,no recurrence was observed. Conclusions Periductal stromal sarcoma（ PDSS） is a low-grade malignant tumor. The treatment is surgery with safe margins,which is particularly important to prevent recurrence. PDSS should not be confused with other spindle cell breast tumors because they require different treatment.